British Journal of Radiology (2006) 79, e205-e207
© 2006 British Institute of Radiology
doi: 10.1259/bjr/12618806
MRI appearances of pelvic malakoplakia
K Gopal, FRCR,
M Dobson, FRCR,
P M Hirsch, FRCS and
H Stringfellow, MRCP(Path)
Lancashire Teaching Hospitals NHS Trust, Royal Preston Hospital, Sharoe Green Lane, Fulwood, Preston PR2 9HT, UK
Correspondence: Dr M Dobson, Consultant Radiologist, Lancashire Teaching Hospitals NHS Trust, Royal Preston Hospital, Sharoe Green Lane, Fulwood, Preston, PR2 9HT, UK. E-mail: mike.dobson{at}lthtr.nhs.uk
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Abstract
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We report the MRI appearances in a patient with parametrial malakoplakia. The patient complained of pelvic pain and vaginal discharge. Physical examination revealed a "frozen" pelvis suggestive of malignancy. MRI showed bilateral parametrial "infiltration", but no overt primary pelvic tumour. The combination of these findings together with the inflammatory symptoms suggested an inflammatory condition. Malakoplakia was confirmed at resective biopsy.
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Introduction
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Malakoplakia is a rare granulomatous inflammatory disease that usually affects the genitourinary tract. In a review of 153 cases in 1981, 58% had involvement of the urinary tract [1]. It often mimics malignancy and may also affect other organs including the gastrointestinal tract, bone, lungs, lymph nodes and skin. The aetiology of the disease is unclear, although most patients have some form of immunosuppression related to organ transplantation, autoimmune diseases requiring steroid use, or chemotherapy [2]. It has also been reported in association with HIV/AIDS [3]. The final diagnosis is histological (see below). Affected patients may develop mass-like inflammatory change causing a clinical suspicion of malignancy. We describe the pelvic MR appearances in such a case.
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Case report
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A 64-year-old female was admitted complaining of right flank and pelvic pain, watery vaginal discharge and mild post-menopausal bleeding for 2 months. Vaginal examination confirmed a profuse watery discharge. The uterus was fixed and very tender. Culture for Chlamydia, gonococcus and yeasts were negative. MR examination revealed extensive bilateral parametrial thickening, extending to the pelvic sidewalls. This was manifest as an "infiltrative" pattern of intermediate signal intensity on T1 and T2 weighted sequences, but with no discrete mass lesion demonstrated (
Figures 1–3). There was also a mild right hydronephrosis and a congenitally absent left kidney. The uterus, bladder and pelvic bowel were normal. Neither ovary was clearly defined, which was not unexpected given the patient's age. Fluid was seen in the vagina, which, in the absence of overt fistulation, lent weight to the suggestion of pelvic inflammatory disease. The appearances were considered non-specific, although suggestive of pelvic actinomycosis. This was the working radiological diagnosis, although there was no history of an intrauterine contraceptive device. The patient underwent a laparotomy, which showed extensive periuterine fibrosis and induration, although no overtly active sepsis. The surgeon maintained a strong suspicion of malignancy at time of operation, and therefore performed a total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH/BSO) and parametrial biopsies. Histology showed extensive parametrial malakoplakia and no evidence of malignancy. The patient was commenced on a prolonged course of antibiotics and made an uneventful recovery. There was no evidence of recurrence on the follow up MR scan 5 months later.
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Figure 1. T1 weighted axial MR images demonstrating extensive, intermediate signal thickening of the parametrial soft tissues (arrows).
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Figure 2. (a,b) T2 weighted axial image demonstrating intermediate signal infiltrative change in the parametria (black arrows). The uterus (white arrow) is intrinsically normal, though is tethered to the right pelvic side wall.
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Figure 3. T2 weighted coronal MR image again demonstrating extensive thickening of the parametria (black arrows). Note the high signal fluid within the vagina (white arrow).
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Discussion
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Malakoplakia is an inflammatory condition thought to arise because of an abnormal response to infection, involving defective lysosomes and an abnormal microtubular assembly [4]. The condition was first described by Michaelis and Gutmann in 1902 and subsequently named by Von Hansemann in 1903 [4]. It may affect many organ systems, although the genitourinary tract is the most common site, most prevalent in women over the age of 50 years. 80–90% of patients have a persistent coliform infection [1] and most patients have some form of immunosuppression such as organ transplantation, chemotherapy or prolonged use of corticosteroids. Less severe forms of immunosuppression such as alcohol abuse, malignancy, poorly controlled diabetes or chronic systemic disease have also been implicated [1, 5].
Macroscopically, the disease consists of soft yellow plaques and nodules usually less than 1 cm in diameter, but ranging up to 3–4 cm, with some progressing to a large sessile mass (as in our case). Histologically, the process is characterized by conglomerates of foamy histiocytes or von Hansemann cells, containing distinctive basophilic inclusion bodies called Michaelis-Gutmann bodies [6]. These bodies are pathognomonic for the disease and help to differentiate it from two similar processes – megalocytic interstitial nephritis and xanthogranulomatous pyelonephritis [7].
Presentation depends upon the organ involved. Malakoplakia of the female urogenital tract commonly presents with haematuria or vaginal bleeding [8]. Biopsy is required for final diagnosis. The presence of large mass-like disease may require surgical resection, especially in the case of ureteric or bowel obstruction. The mainstay of treatment is long-term antibiotic therapy to render the urine sterile [1]. Quinolones are effective in 80–90% of patients although other agents such as trimethoprim and rifampicin are useful. If possible, immunosuppressive drugs should be stopped [9].
To our knowledge, MRI appearances of pelvic malakoplakia have not been described previously. The striking feature in our case was extensive abnormal thickening of the parametrial tissues with no overt intrinsic abnormality of the uterus, bladder or rectum. A similar appearance has been described in cases of pelvic actinomycosis [10], although the latter is almost always associated with the use of an intrauterine contraceptive device [10], which was not a factor for our patient. The presence of the features described should raise the possibility of malakoplakia. Based on such findings, it would be reasonable for the radiologist to urge an initially conservative approach to management, with a follow up MR scan recommended after a protracted course of antibiotics.
Received for publication July 22, 2005.
Revision received February 2, 2006.
Accepted for publication February 3, 2006.
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References
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Abstract
Introduction
